Charcot-Marie-Tooth Disease (CMT)

Anesthesia Implications

Anesthesia Implications

Malignant hyperthermia (MH) risk – This is widely debated. One study showed a patient that developed MH, so use of succinylcholine is generally discouraged. Multiple studies have shown the use of inhaled anesthetics as safe, though because of the association, other means of anesthetic maintenance should be explored.

Positioning – these patients may have limb abnormalities/deformities that make positioning difficult.

Respiratory weakness – assess and document carefully the respiratory function of the patient. Diaphragmatic dysfunction may be present and would warrant consideration of prolonged ventilation postoperatively.

Paralytics – avoid the use of succinylcholine. Nondepolarizing muscle relaxants (eg Rocuronium) will have prolonged effects.

Local anesthetics – these will have prolonged/exaggerated effects


This condition is inherited and the most common cause of chronic motor and sensory peripheral neuropathy. It is characterized by progressive motor-sensory disorders in all 4 limbs. Motor weakness eventually progresses to muscle atrophy and can lead chronic muscular dystrophy. Affects males more than females.

Type 1 – autosomal dominant. Characterized by nerve demyelination.
Type 2 – autosomal dominant. Characterized by axonal dysfunction.
Type 3 – autosomal recessive. Rare.
Type 4 – autosomal recessive. Rare.

Symptoms – atrophy of peroneal muscles and motor-sensory abnormalities in all four limbs. Typically manifests in the mid-teens. Weakness typically starts in the lower limbs and progresses to the upper limbs. Autonomic function is also often affected. The disease progress varies widely. Dysrhythmias may be present and vary as well. Reports show possibilities of complete right bundle branch block, mitral valve prolapse with second-degree atrioventricular block, third-degree atrioventricular block, sick sinus syndrome requiring pacemaker insertion, and paroxysmal atrial flutter.


Hines. Stoelting’s anesthesia and co-existing disease. 7th edition. 2018.
Malcom. Anesthesia. 2020 link
Ohshita. Anesthetic management of a patient with Charcot-Marie-Tooth disease. 2016 link